bims-malgli Biomed News
on Biology of malignant gliomas
Issue of 2023–06–18
sixteen papers selected by
Oltea Sampetrean, Keio University



  1. Acta Neuropathol Commun. 2023 Jun 16. 11(1): 96
      Pediatric high-grade gliomas (pHGG) are brain tumors occurring in children and adolescents associated with a dismal prognosis despite existing treatments. Therapeutic failure in both adult and pHGG has been partially imputed to glioma stem cells (GSC), a subset of cancer cells endowed with stem-like cell potential and malignant, invasive, adaptative, and treatment-resistant capabilities. Whereas GSC have largely been portrayed in adult tumors, less information has been provided in pHGG. The aim of our study was to comprehensively document the stem-like capacities of seven in-use pediatric glioma cell cultures (Res259, UW479, SF188, KNS42, SF8628, HJSD-DIPG-007 and HJSD-DIPG-012) using parallel in vitro assays assessing stem cell-related protein expression, multipotency, self-renewal and proliferation/quiescence, and in vivo investigation of their tumorigenicity and invasiveness. Data obtained from in vitro experiments revealed glioma subtype-dependent expression of stem cell-related markers and varying abilities for differentiation, self-renewal, and proliferation/quiescence. Among tested cultures, DMG H3-K27 altered cultures displayed a particular pattern of stem-like markers expression and a higher fraction of cells with self-renewal potential. Four cultures displaying distinctive stem-like profiles were further tested for their ability to initiate tumors and invade the brain tissue in mouse orthotopic xenografts. The selected cell cultures all showed a great tumor formation capacity, but only DMG H3-K27 altered cells demonstrated a highly infiltrative phenotype. Interestingly, we detected DMG H3-K27 altered cells relocated in the subventricular zone (SVZ), which has been previously described as a neurogenic area, but also a potential niche for brain tumor cells. Finally, we observed an SVZ-induced phenotypic modulation of the glioma cells, as evidenced by their increased proliferation rate. In conclusion, this study recapitulated a systematic stem-like profiling of various pediatric glioma cell cultures and call to a deeper characterization of DMG H3-K27 altered cells nested in the SVZ.
    Keywords:  Children; Diffuse infiltrating pontine glioma; Diffuse midline glioma; Glioblastoma; Glioma stem cells; High-grade glioma; Histone; Pediatric; Slow-cycling cells; Subventricular zone
    DOI:  https://doi.org/10.1186/s40478-023-01586-x
  2. Cancers (Basel). 2023 May 25. pii: 2902. [Epub ahead of print]15(11):
      High-grade gliomas are malignant brain tumors, and patient outcomes remain dismal despite the emergence of immunotherapies aimed at promoting tumor elimination by the immune system. A robust antitumor immune response requires the presentation of tumor antigens by dendritic cells (DC) to prime cytolytic T cells. However, there is a paucity of research on dendritic cell activity in the context of high-grade gliomas. As such, this review covers what is known about the role of DC in the CNS, DC infiltration of high-grade gliomas, tumor antigen drainage, the immunogenicity of DC activity, and DC subsets involved in the antitumor immune response. Finally, we consider the implications of suboptimal DC function in the context of immunotherapies and identify opportunities to optimize immunotherapies to treat high-grade gliomas.
    Keywords:  T cell priming; dendritic cells; glioblastoma; glioma; immunotherapy
    DOI:  https://doi.org/10.3390/cancers15112902
  3. Redox Biol. 2023 Jun 05. pii: S2213-2317(23)00174-X. [Epub ahead of print]64 102773
       PURPOSE: Glioblastoma (GBM) is the most common type of adult brain tumor with extremely poor survival. Cystathionine-gamma lyase (CTH) is one of the main Hydrogen Sulfide (H2S) producing enzymes and its expression contributes to tumorigenesis and angiogenesis but its role in glioblastoma development remains poorly understood.
    METHODS: and Principal Results: An established allogenic immunocompetent in vivo GBM model was used in C57BL/6J WT and CTH KO mice where the tumor volume and tumor microvessel density were blindly measured by stereological analysis. Tumor macrophage and stemness markers were measured by blinded immunohistochemistry. Mouse and human GBM cell lines were used for cell-based analyses. In human gliomas, the CTH expression was analyzed by bioinformatic analysis on different databases. In vivo, the genetic ablation of CTH in the host led to a significant reduction of the tumor volume and the protumorigenic and stemness transcription factor sex determining region Y-box 2 (SOX2). The tumor microvessel density (indicative of angiogenesis) and the expression levels of peritumoral macrophages showed no significant changes between the two genotypes. Bioinformatic analysis in human glioma tumors revealed that higher CTH expression is positively correlated to SOX2 expression and associated with worse overall survival in all grades of gliomas. Patients not responding to temozolomide have also higher CTH expression. In mouse or human GBM cells, pharmacological inhibition (PAG) or CTH knockdown (siRNA) attenuates GBM cell proliferation, migration and stem cell formation frequency.
    MAJOR CONCLUSIONS: Inhibition of CTH could be a new promising target against glioblastoma formation.
    Keywords:  Brain blood vessels; Cystathionine gamma-lyase (CTH); Glioblastoma stem cells (GSC); Sex determining region Y-Box 2 (SOX2)
    DOI:  https://doi.org/10.1016/j.redox.2023.102773
  4. Neuro Oncol. 2023 Jun 16. pii: noad107. [Epub ahead of print]
      Glioblastoma (GBM) is the most prevalent malignant primary brain tumor, accounting for 14.2% of all diagnosed tumors and 50.1% of all malignant tumors, and the median survival time is approximately 8 months irrespective of whether a patient receives treatment without significant improvement despite expansive research 1. Recently, important roles for the circadian clock in GBM tumorigenesis have been reported. Positive regulators of circadian-controlled transcription, Brain and Muscle ARNT-Like 1 (BMAL1) and Circadian Locomotor Output Cycles Kaput (CLOCK), are highly expressed also in GBM and correlated with poor patient prognosis. BMAL1 and CLOCK promote maintenance of GBM stem cells (GSCs) and establishment of a pro-tumorigenic tumor microenvironment (TME), suggesting that targeting the core clock proteins may augment GBM treatment. Here, we review findings that highlight the critical role the circadian clock plays in GBM biology and the strategies by which the circadian clock can be leveraged for GBM treatment in the clinic moving forward.
    Keywords:  Chronomedicine; Chronotherapy; Circadian Pharmacology; Circadian Rhythm; Glioblastoma
    DOI:  https://doi.org/10.1093/neuonc/noad107
  5. Neurooncol Adv. 2023 Jan-Dec;5(1):5(1): vdad066
       Background: Although the epidermal growth factor receptor (EGFR) is a frequent oncogenic driver in glioblastoma (GBM), efforts to therapeutically target this protein have been largely unsuccessful. The present preclinical study evaluated the novel EGFR inhibitor WSD-0922.
    Methods: We employed flank and orthotopic patient-derived xenograft models to characterize WSD-0922 and compare its efficacy to erlotinib, a potent EGFR inhibitor that failed to provide benefit for GBM patients. We performed long-term survival studies and collected short-term tumor, plasma, and whole-brain samples from mice treated with each drug. We utilized mass spectrometry to measure drug concentrations and spatial distribution and to assess the impact of each drug on receptor activity and cellular signaling networks.
    Results: WSD-0922 inhibited EGFR signaling as effectively as erlotinib in in vitro and in vivo models. While WSD-0922 was more CNS penetrant than erlotinib in terms of total concentration, comparable concentrations of both drugs were measured at the tumor site in orthotopic models, and the concentration of free WSD-0922 in the brain was significantly less than the concentration of free erlotinib. WSD-0922 treatment provided a clear survival advantage compared to erlotinib in the GBM39 model, with marked suppression of tumor growth and most mice surviving until the end of the study. WSD-0922 treatment preferentially inhibited phosphorylation of several proteins, including those associated with EGFR inhibitor resistance and cell metabolism.
    Conclusions: WSD-0922 is a highly potent inhibitor of EGFR in GBM, and warrants further evaluation in clinical studies.
    Keywords:  EGFR; WSD-0922; blood-brain barrier; erlotinib; glioblastoma
    DOI:  https://doi.org/10.1093/noajnl/vdad066
  6. Brain Commun. 2023 ;5(3): fcad176
      Glioblastoma is a devastating brain cancer for which effective treatments are required. Tumour-associated microglia and macrophages promote glioblastoma growth in an immune-suppressed microenvironment. Most recurrences occur at the invasive margin of the surrounding brain, yet the relationships between microglia/macrophage phenotypes, T cells and programmed death-ligand 1 (an immune checkpoint) across human glioblastoma regions are understudied. In this study, we performed a quantitative immunohistochemical analysis of 15 markers of microglia/macrophage phenotypes (including anti-inflammatory markers triggering receptor expressed on myeloid cells 2 and CD163, and the low-affinity-activating receptor CD32a), T cells, natural killer cells and programmed death-ligand 1, in 59 human IDH1-wild-type glioblastoma multi-regional samples (n = 177; 1 sample at tumour core, 2 samples at the margins: the infiltrating zone and leading edge). Assessment was made for the prognostic value of markers; the results were validated in an independent cohort. Microglia/macrophage motility and activation (Iba1, CD68), programmed death-ligand 1 and CD4+ T cells were reduced, and homeostatic microglia (P2RY12) were increased in the invasive margins compared with the tumour core. There were significant positive correlations between microglia/macrophage markers CD68 (phagocytic)/triggering receptor expressed on myeloid cells 2 (anti-inflammatory) and CD8+ T cells in the invasive margins but not in the tumour core (P < 0.01). Programmed death-ligand 1 expression was associated with microglia/macrophage markers (including anti-inflammatory) CD68, CD163, CD32a and triggering receptor expressed on myeloid cells 2, only in the leading edge of glioblastomas (P < 0.01). Similarly, there was a positive correlation between programmed death-ligand 1 expression and CD8+ T-cell infiltration in the leading edge (P < 0.001). There was no relationship between CD64 (a receptor for autoreactive T-cell responses) and CD8+/CD4+ T cells, or between the microglia/macrophage antigen presentation marker HLA-DR and microglial motility (Iba1) in the tumour margins. Natural killer cell infiltration (CD335+) correlated with CD8+ T cells and with CD68/CD163/triggering receptor expressed on myeloid cells 2 anti-inflammatory microglia/macrophages at the leading edge. In an independent large glioblastoma cohort with transcriptomic data, positive correlations between anti-inflammatory microglia/macrophage markers (triggering receptor expressed on myeloid cells 2, CD163 and CD32a) and CD4+/CD8+/programmed death-ligand 1 RNA expression were validated (P < 0.001). Finally, multivariate analysis showed that high triggering receptor expressed on myeloid cells 2, programmed death-ligand 1 and CD32a expression at the leading edge were significantly associated with poorer overall patient survival (hazard ratio = 2.05, 3.42 and 2.11, respectively), independent of clinical variables. In conclusion, anti-inflammatory microglia/macrophages, CD8+ T cells and programmed death-ligand 1 are correlated in the invasive margins of glioblastoma, consistent with immune-suppressive interactions. High triggering receptor expressed on myeloid cells 2, programmed death-ligand 1 and CD32a expression at the human glioblastoma leading edge are predictors of poorer overall survival. Given substantial interest in targeting microglia/macrophages, together with immune checkpoint inhibitors in cancer, these data have major clinical implications.
    Keywords:  glioblastoma; immunotherapy; intratumoural heterogeneity; macrophage; microglia
    DOI:  https://doi.org/10.1093/braincomms/fcad176
  7. Front Immunol. 2023 ;14 1065062
       Background: Siglec15 is rising as a promising immunotherapeutic target in bladder, breast, gastric, and pancreatic cancers. The aim of the present study is to explore the prognostic value and immunotherapeutic possibilities of Siglec15 in gliomas using bioinformatics and clinicopathological methods.
    Methods: The bioinformatics approach was used to examine Siglec15 mRNA expression in gliomas based on TCGA, CGGA, and GEO datasets. Then, the predictive value of Siglec15 expression on progression-free survival time (PFST) and overall survival time (OST) in glioma patients was comprehensively described.The TCGA database was screened for differentially expressed genes (DEGs) between the high and low Siglec15 expression groups, and enrichment analysis of the DEGs was performed. The Siglec15 protein expression and its prognostic impact in 92 glioma samples were explored using immunohistochemistry Next, the relationships between Siglec15 expression and infiltrating immune cells, immune regulators and multiple immune checkpoints were analysed.
    Results: Bioinformatics analyses showed that high Siglec15 levels predicted poor clinical prognosis and adverse recurrence time in glioma patients. In the immunohistochemical study serving as a validation set, Siglec15 protein overexpression was found in 33.3% (10/30) of WHO grade II, 56% (14/25) of WHO grade III, and 70.3% (26/37) of WHO grade IV gliomas respectively. Siglec15 protein overexpression was also found to be an independent prognostic indicator detrimental to the PFST and OST of glioma patients. Enrichment analysis showed that the DEGs were mainly involved in pathways associated with immune function, including leukocyte transendothelial migration, focal adhesion, ECM receptor interaction, and T-cell receptor signaling pathways. In addition, high Siglec15 expression was related to M2 tumor-associated macrophages (TAMs), N2 tumor-infiltrating neutrophils, suppressive tumor immune microenvironment, and multiple immune checkpoint molecules. Immunofluorescence analysis confirmed the colocalization of Siglec15 and CD163 on TAMs.
    Conclusion: Siglec15 overexpression is common in gliomas and predicts an adverse recurrence time and overall survival time. Siglec15 is a potential target for immunotherapy and a potential TAMs regulator that is involved in the suppressed immunomicroenvironment in gliomas.
    Keywords:  SIGLEC15; gliomas; immune checkpoint; macrophages; prognostic indicator
    DOI:  https://doi.org/10.3389/fimmu.2023.1065062
  8. Clin Cancer Res. 2023 Jun 16. pii: CCR-23-0203. [Epub ahead of print]
       PURPOSE: We evaluated the efficacy of bavituximab - a monoclonal antibody with anti-angiogenic and immunomodulatory properties - in newly diagnosed glioblastoma (GBM) patients who also received radiation and temozolomide. Perfusion MRI and myeloid-related gene transcription and inflammatory infiltrates in pre-and post-treatment tumor specimens were studied to evaluate on-target effects (NCT03139916).
    PATIENTS AND METHODS: Thirty-three adults with isocitrate-dehydrogenase-wild-type GBM received 6 weeks of concurrent chemoradiation, followed by 6 cycles of temozolomide (C1-C6). Bavituximab was given weekly, starting week 1 of chemoradiation, for at least 18 weeks. The primary endpoint was proportion of patients alive at 12 months (OS-12). The null hypothesis would be rejected if OS-12 was ≥72%. Relative cerebral blood flow (rCBF) and vascular permeability (Ktrans) were calculated from perfusion MRIs. Peripheral blood mononuclear cells (PBMCs) and tumor tissue were analyzed pre-treatment and at disease progression using RNA transcriptomics and multispectral immunofluorescence for myeloid-derived suppressor cells (MDSCs) and macrophages.
    RESULTS: The study met its primary endpoint with an OS-12 of 73% (95% CI 59-90%). Decreased pre-C1 rCBF (HR 4.63, p=0.029) and increased pre-C1 Ktrans were associated with improved OS (HR 0.09, p=0.005). Pre-treatment, overexpression of myeloid-related genes in tumor tissue was associated with longer survival. Post-treatment, tumor specimens contained fewer immunosuppressive MDSCs (p=0.01).
    DISCUSSION: Bavituximab has activity in newly diagnosed GBM and resulted in on-target depletion of intratumoral immunosuppressive MDSCs. Elevated pre-treatment expression of myeloid-related transcripts in GBM may predict response to bavituximab.
    DOI:  https://doi.org/10.1158/1078-0432.CCR-23-0203
  9. Oncogene. 2023 Jun 15.
      Gliomas are highly malignant tumors accounting for the majority of brain neoplasms. They are characterized by nuclear atypia, high mitotic rate and cellular polymorphism that often contributes to aggressiveness and resistance to standard therapy. They often associate with challenging treatment approaches and poor outcomes. New treatment strategies or regimens to improve the efficacy of glioma treatment require a deeper understanding of glioma occurrence and development as well as elucidation of their molecular biological characteristics. Recent studies have revealed RNA modifications as a key regulatory mechanism involved in tumorigenesis, tumor progression, immune regulation, and response to therapy. The present review discusses research advances on several RNA modifications involved in glioma progression and tumor microenvironment (TME) immunoregulation as well as in the development of adaptive drug resistance, summarizing current progress on major RNA modification targeting strategies.
    DOI:  https://doi.org/10.1038/s41388-023-02746-y
  10. Neurooncol Adv. 2023 Jan-Dec;5(1):5(1): vdad069
       Background: Isocitrate dehydrogenase (IDH) mutations are thought to represent an early oncogenic event in glioma evolution, found with high penetrance across tumor cells; however, in rare cases, IDH mutation may exist only in a small subset of the total tumor cells (subclonal IDH mutation).
    Methods: We present 2 institutional cases with subclonal IDH1 R132H mutation. In addition, 2 large publicly available cohorts of IDH-mutant astrocytomas were mined for cases harboring subclonal IDH mutations (defined as tumor cell fraction with IDH mutation ≤0.67) and the clinical and molecular features of these subclonal cases were compared to clonal IDH-mutant astrocytomas.
    Results: Immunohistochemistry (IHC) performed on 2 institutional World Health Organization grade 4 IDH-mutant astrocytomas revealed only a minority of tumor cells in each case with IDH1 R132H mutant protein, and next-generation sequencing (NGS) revealed remarkably low IDH1 variant allele frequencies compared to other pathogenic mutations, including TP53 and/or ATRX. DNA methylation classified the first tumor as high-grade IDH-mutant astrocytoma with high confidence (0.98 scores). In the publicly available datasets, subclonal IDH mutation was present in 3.9% of IDH-mutant astrocytomas (18/466 tumors). Compared to clonal IDH-mutant astrocytomas (n = 156), subclonal cases demonstrated worse overall survival in grades 3 (P = .0106) and 4 (P = .0184).
    Conclusions: While rare, subclonal IDH1 mutations are present in a subset of IDH-mutant astrocytomas of all grades, which may lead to a mismatch between IHC results and genetic/epigenetic classification. These findings suggest a possible prognostic role of IDH mutation subclonality, and highlight the potential clinical utility of quantitative IDH1 mutation evaluation by IHC and NGS.
    Keywords:  IDH mutation; astrocytoma; glioblastoma; mosaic IDH1 R132H immunohistochemistry; oligodendroglioma; subclonal mutation
    DOI:  https://doi.org/10.1093/noajnl/vdad069
  11. Neurooncol Adv. 2023 Jan-Dec;5(1):5(1): vdad072
      [This corrects the article DOI: 10.1093/noajnl/vdac079.].
    DOI:  https://doi.org/10.1093/noajnl/vdad072
  12. J Math Biol. 2023 Jun 10. 87(1): 4
      Pseudopalisading is an interesting phenomenon where cancer cells arrange themselves to form a dense garland-like pattern. Unlike the palisade structure, a similar type of pattern first observed in schwannomas by pathologist J.J. Verocay (Wippold et al. in AJNR Am J Neuroradiol 27(10):2037-2041, 2006), pseudopalisades are less organized and associated with a necrotic region at their core. These structures are mainly found in glioblastoma (GBM), a grade IV brain tumor, and provide a way to assess the aggressiveness of the tumor. Identification of the exact bio-mechanism responsible for the formation of pseudopalisades is a difficult task, mainly because pseudopalisades seem to be a consequence of complex nonlinear dynamics within the tumor. In this paper we propose a data-driven methodology to gain insight into the formation of different types of pseudopalisade structures. To this end, we start from a state of the art macroscopic model for the dynamics of GBM, that is coupled with the dynamics of extracellular pH, and formulate a terminal value optimal control problem. Thus, given a specific, observed pseudopalisade pattern, we determine the evolution of parameters (bio-mechanisms) that are responsible for its emergence. Random histological images exhibiting pseudopalisade-like structures are chosen to serve as target pattern. Having identified the optimal model parameters that generate the specified target pattern, we then formulate two different types of pattern counteracting ansatzes in order to determine possible ways to impair or obstruct the process of pseudopalisade formation. This provides the basis for designing active or live control of malignant GBM. Furthermore, we also provide a simple, yet insightful, mechanism to synthesize new pseudopalisade patterns by linearly combining the optimal model parameters responsible for generating different known target patterns. This particularly provides a hint that complex pseudopalisade patterns could be synthesized by a linear combination of parameters responsible for generating simple patterns. Going even further, we ask ourselves if complex therapy approaches can be conceived, such that some linear combination thereof is able to reverse or disrupt simple pseudopalisade patterns; this is investigated with the help of numerical simulations.
    Keywords:  Data driven modeling; Optimal control; Pattern formation; Pseudopalisades
    DOI:  https://doi.org/10.1007/s00285-023-01933-5
  13. Neuro Oncol. 2023 Jun 15. pii: noad105. [Epub ahead of print]
       OBJECTIVE: To determine the safety, tolerability and distribution of MTX110 (aqueous panobinostat) delivered by convection enhanced delivery (CED) in patients with newly diagnosed diffuse intrinsic pontine glioma (DIPG) who completed focal radiation therapy (RT).
    METHODS: Patients with DIPG (2-21 years) were enrolled after RT. CED of MTX110 combined with gadoteridol was completed across seven dose levels (DL) (30-90 µM; volumes ranging from 3 mL to two consecutive doses of 6 mL). An accelerated dose escalation design was used. Distribution of infusate was monitored with real-time MR imaging. Repeat CED was performed every 4-8 weeks. Quality of life (QOL) assessments were obtained at baseline, every 3 months on therapy, and end of therapy.
    RESULTS: Between May 2018-March 2020 seven patients, who received a total of 48 CED infusions, were enrolled (median age 8 years, range 5-21). Three patients experienced dose-limited toxicities. Four grade 3 treatment related adverse events were observed. Most toxicities were transient new or worsening neurologic function. Median overall survival (OS) was 26.1 months (95% CI: 14.8-not reached). Progression free survival was 4-14 months (median, 7). Cumulative percentage of tumor coverage for combined CED infusions per patient ranged from 35.6-81.0%. Increased CED infusions was negatively associated with self-reported QOL assessments.
    CONCLUSION: Repeat CED of MTX110 with real time imaging with gadoteridol is tolerable for patients with DIPG. Median OS of 26.1 months compares favorably with historical data for children with DIPG. The results support further investigation of this strategy in a larger cohort.
    Keywords:  DIPG; MTX110; convection enhanced delivery; real time imaging monitoring
    DOI:  https://doi.org/10.1093/neuonc/noad105
  14. Nat Med. 2023 Jun 12.
      
    Keywords:  CNS cancer; Cancer therapy; Clinical trials; Targeted therapies
    DOI:  https://doi.org/10.1038/d41591-023-00054-2